Elisa Listo^1,#, Nicola Martini^2,#, Stefano Salvadori³, Elisa Valenti³, Nicola Stagnaro¹, Gianluca Trocchio⁴, Chiara Marrone⁵, Alberto Clemente⁶, Francesca Raimondi^7,*, Pierluigi Festa⁵, Lamia Ait Ali^5,8,*

Congenital Heart Disease, Vol.18, No.6, pp. 627-638, 2023, DOI:10.32604/chd.2023.030312

Abstract Background: The assessment of Fontan circuit’s flow is traditionally evaluated by multiple through-plane phase-contrast MRI acquisitions (2D flow), while recently, a single volumetric 4D-flow MRI acquisition is emerging as a comprehensive tool for the hemodynamic evaluation in congenital heart diseases. Purpose: To compare 2D and 4D-flow MRI measurements in patients after Fontan palliation and to evaluate parameters affecting potential disagreement. Methods: 39 patients after Fontan palliation (23 males, age 22 ± 11 years) who underwent cardiac MRI with 2D and 4D-flow MRI acquisition were included in the study. In all patients, blood flow quantification in the Fontan circuit and aorta… More > Graphic Abstract

Charis Tan^1,2 , Diana Zannino³, Carley Clendenning³, Sophie Offen⁴, Thomas L. Gentles⁵, Julian Ayer⁶, David Tanous⁷, Vishva Wijesekera⁸, Leeanne Grigg⁹, David Celermajer^2,4,10, Mark McGuire^2,4 , Yves d’Udekem^3,11,12, Rachael Cordina^2,4,10,*

Congenital Heart Disease, Vol.18, No.5, pp. 507-523, 2023, DOI:10.32604/chd.2023.028829

Abstract Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA; 14 (1.0%) had… More >

Nerejda Shehu^1,*, Christian Meierhofer¹, Anja Hennemuth^2,3, Markus Hüllebrand^2,3, Pavlo Yevtushenko³, Peter Ewert¹, Stefan Martinoff⁴, Heiko Stern¹

Congenital Heart Disease, Vol.18, No.4, pp. 461-474, 2023, DOI:10.32604/chd.2023.031075

Abstract Background: Pressure measurement in total cavopulmonary connection (TCPC) patients is a domain of cardiac catheterization. 4D velocity encoded cardiovascular magnetic resonance (4D–flow MRI) offers an alternative for assessment of even minor pressure differences. The scope of this study was to measure even minor pressure differences in the anastomosis of TCPC patients, who are clinically uncompromised. Methods: Twenty-four patients (median 15 years [8;34]) with TCPC were studied prospectively by 4D-flow MRI. Pressure differences between superior vena cava (SVC) and extracardiac conduit (C) to both right pulmonary artery (RPA) and left pulmonary artery (LPA) were assessed. Small fluid obstructions as vortices within… More > Graphic Abstract

Yuki Kawasaki^1,*, Takeshi Sasaki¹, Daisuke Kobayashi²

Congenital Heart Disease, Vol.18, No.4, pp. 431-445, 2023, DOI:10.32604/chd.2023.030910

Abstract Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized that hemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinical features and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients who underwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI  ≥ 3, SVR index (SVRI) ≥ 20; B, CI < 3, SVRI ≥ 20; C, CI ≥ 3, SVRI < 20; and D, CI < 3, SVRI < 20. The primary outcome was freedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant, and death. Clinical and hemodynamic variables and freedom from… More > Graphic Abstract

Jinlong Liu^1,2,#, Jing Shi^3,#, Weiru Luo¹, Zhirong Tong^1,2, Lefei Yang³, Peixuan Sun³, Tianyi Li³, Jun Du^3,*, Qian Wang^3,*

Congenital Heart Disease, Vol.18, No.1, pp. 41-55, 2023, DOI:10.32604/chd.2023.025005

Abstract Background: The assessment of renal function is important to the prognosis of patients needing Fontan palliation due to the reconstructed compromised circulation. To know the relationship between the kidney perfusion and hemodynamic characteristics during surgical design could reduce the risk of acute kidney injury (AKI) and the postoperative complications. However, the issue is still unsolved because the current clinical evaluation methods are unable to predict the hemodynamic changes in renal artery (RA). Methods: We reconstructed a three-dimensional (3D) vascular model of a patient requiring Fontan palliation. The technique of computational fluid dynamics (CFD) was utilized to explore the changes of… More > Graphic Abstract

Emma Payne^1,2, Thomas Wilson^2,3, Marjan Haghighi^1,4, Zoe McCallum^2,3, Yves d’Udekem⁵, Julian Ayer^1,4,*

Congenital Heart Disease, Vol.17, No.6, pp. 617-639, 2022, DOI:10.32604/chd.2022.024775

Abstract Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies that underwent title… More >

Eleni P. Asimacopoulos^*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

Congenital Heart Disease, Vol.17, No.5, pp. 579-590, 2022, DOI:10.32604/chd.2022.020334

Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study to have a factor… More >

Ju Wang, Shuo Dong, Jun Yan^*

Congenital Heart Disease, Vol.16, No.5, pp. 477-485, 2021, DOI:10.32604/CHD.2021.015016

Abstract Objective: Modifications of the Fontan operation, which are also known as total cavopulmonary connection (TCPC), are widely applied for patients with functionally univentricular hearts (FUH). Herein, we summed up the different surgical pathways and clinical outcomes in FUH patients with apicocaval juxtaposition (ACJ) or/and separated hepatic venous (SHV) drainage. Methods: Between January 2009 and December 2019, 123 patients who undergone TCPC in our institute were included in this retrospective study. We have included 70 patients with ACJ (Group 1) and 53 patients with SHV (Group 2). Moreover, Group 2 included 17 cases combing with ACJ (32.1%). In Group 1, three… More >

Pietro Paolo Tamborrino^1,2, Corrado Di Mambro^1,2,*, Cecilia Marcolin^1,2, Walter Vignaroli³, Giulia Cafiero⁴, Gianluca Brancaccio³, Sonia Albanese³, Massimo Stefano Silvetti^1,2, Adriano Carotti³, Fabrizio Drago^1,2

Congenital Heart Disease, Vol.16, No.1, pp. 85-94, 2021, DOI:10.32604/CHD.2021.013038

Abstract Introduction: While previous studies only focused on the arrhythmic risk associated with specific correction strategies, this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair. Methods: In this single centre observational cohort study, we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment, patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes. Surgical history and clinical outcomes were reviewed. Results: 86 patients were included in the study (54 male [62.8%], mean age 16.4 ± 6.1 years), with… More >

Sheetal R. Patel^1,2,*, David M. Kwiatkowski³, Adin-Cristian Andrei², Ankita Devareddy², Hangzhi Shi², Catherine D. Krawczeski⁴, Natalie Ebert⁵, Barbara J. Deal^1,2, Craig B. Langman^2,6, Bradley S. Marino^1,2

Congenital Heart Disease, Vol.15, No.4, pp. 181-195, 2020, DOI:10.32604/CHD.2020.012097

Abstract Objectives: The Fontan operation has increased survival in patients with single-ventricle congenital heart defects. However, Fontan survivors are at risk of other organ dysfunctions, such as renal dysfunction (RD). The objectives of this study are to assess the prevalence of and potential risk factors for RD among Fontan survivors. Design, setting, and patients: We performed a two-center, cross-sectional study that included Fontan survivors evaluated in outpatient-clinics for routine follow up between 01/08-12/16. Outcome measures: The primary outcome was the presence of RD defined by estimated glomerular filtration rate (eGFR) of <90 ml/min/1.73 m² derived using the serum creatinine-based Full Age… More >